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Towards the development of an enzyme replacement therapy for the metabolic disorder propionic acidemia
Propionic acidemia (PA) is a life-threatening disease caused by the deficiency of a mitochondrial biotin-dependent enzyme known as propionyl coenzyme-A carboxylase (PCC). This enzyme is responsible for degrading the metabolic intermediate, propionyl coenzyme-A (PP-CoA), derived from multiple metabol...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Elsevier
2016
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4968140/ https://www.ncbi.nlm.nih.gov/pubmed/27504265 http://dx.doi.org/10.1016/j.ymgmr.2016.06.009 |